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Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups.

ABSTRACT: BACKGROUND:Systemic lupus erythematous (SLE) is a systemic autoimmune/inflammatory condition. Approximately 15-20% of patients develop symptoms before their 18th birthday and are diagnosed with juvenile-onset SLE (JSLE). Gender distribution, clinical presentation, disease courses and outcomes vary significantly between JSLE patients and individuals with adult-onset SLE. This study aimed to identify age-specific clinical and/or serological patterns in JSLE patients enrolled to the UK JSLE Cohort Study. METHODS:Patient records were accessed and grouped based on age at disease-onset: pre-pubertal (?7 years), peri-pubertal (8-13 years) and adolescent (14-18 years). The presence of American College of Rheumatology (ACR) classification criteria, laboratory results, disease activity [British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2?K) scores] and damage [Systemic Lupus International Collaborating Clinics (SLICC) damage index] were evaluated at diagnosis and last follow up. RESULTS:A total of 418 JSLE patients were included in this study: 43 (10.3%) with pre-pubertal disease onset; 240 (57.4%) with peri-pubertal onset and 135 (32.3%) were diagnosed during adolescence. At diagnosis, adolescent JSLE patients presented with a higher number of ACR criteria when compared with pre-pubertal and peri-pubertal patients [pBILAG2004 scores: 9(4-20] vs. 7(3-13] vs. 7(3-14], respectively, p?=?0.015] with increased activity in the following BILAG domains: mucocutaneous (p?=?0.025), musculoskeletal (p?=?0.029), renal (p?=?0.027) and cardiorespiratory (p?=?0.001). Furthermore, adolescent JSLE patients were more frequently ANA-positive (p?=?0.034) and exhibited higher anti-dsDNA titres (p?=?0.001). Pre-pubertal individuals less frequently presented with leukopenia (p?=?0.002), thrombocytopenia (p?=?0.004) or low complement (p?=?0.002) when compared with other age groups. No differences were identified in disease activity (pBILAG2004 score), damage (SLICC damage index) and the number of ACR criteria fulfilled at last follow up. CONCLUSIONS:Disease presentations and laboratory findings vary significantly between age groups within a national cohort of JSLE patients. Patients diagnosed during adolescence exhibit greater disease activity and "classic" autoantibody, immune cell and complement patterns when compared with younger patients. This supports the hypothesis that pathomechanisms may vary between patient age groups.

SUBMITTER: Massias JS

PROVIDER: S-EPMC7528537 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature

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Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups.

Massias J S JS Smith E M D EMD Al-Abadi E E Armon K K Bailey K K Ciurtin C C Davidson J J Gardner-Medwin J J Haslam K K Hawley D P DP Leahy A A Leone V V McErlane F F Mewar D D Modgil G G Moots R R Pilkington C C Ramanan A V AV Rangaraj S S Riley P P Sridhar A A Wilkinson N N Beresford M W MW Hedrich C M CM

Lupus 20200331 5

<h4>Background</h4>Systemic lupus erythematous (SLE) is a systemic autoimmune/inflammatory condition. Approximately 15-20% of patients develop symptoms before their 18th birthday and are diagnosed with juvenile-onset SLE (JSLE). Gender distribution, clinical presentation, disease courses and outcomes vary significantly between JSLE patients and individuals with adult-onset SLE. This study aimed to identify age-specific clinical and/or serological patterns in JSLE patients enrolled to the UK JSLE ...[more]

PMID: 32233733

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Project description:IntroductionSystemic lupus erythematosus (SLE) is a multisystem autoimmune disease that is associated with significant morbidity and mortality. SLE disproportionately affects women and minorities. Childhood-onset SLE (cSLE) in particular tends to be more aggressive than adult-onset SLE. Despite substantial improvements in the treatment of cSLE, there is significant variability in treatment responses and long-term outcomes. Furthermore, there is a paucity of studies involving cSLE, and in particular, cSLE among different age groups. The aim of this study was to test the hypothesis that an early-onset cSLE cohort would demonstrate unique characteristics with distinctive clinical and laboratory features at disease onset. We specifically investigated whether clinical, epidemiological, or serological factors are differentially associated with early- and late-onset cSLE. This could have direct impact on clinical management with the goal of improving outcomes and quality of life for children with SLE.MethodsOur study was conducted at a large tertiary center. We included 213 subjects seen at our pediatric rheumatology clinic aged 4-17 years. Epidemiologic, clinical phenotype, disease severity, serology, treatment, and outcome data were compared between subjects with cSLE onset prior to 10 years of age (early-onset disease, n = 43) and those with cSLE onset greater than 10 years of age (peri-adolescent disease, n = 170). We compared clinical features between early- and peri-adolescent onset cSLE in order to investigate the association between age at disease onset of cSLE and clinical disease expression and outcomes.ResultsOf the 213 subjects with cSLE in our study, 43 subjects had early-onset disease (age 2 to ≤9 years) and 170 patients had peri-adolescent onset disease. We found that early-onset cSLE was associated with a higher prevalence of positive anti-dsDNA antibody at cSLE diagnosis, higher anti-dsDNA antibody titer at cSLE diagnosis, rash, and azathioprine use (p < 0.001, p = 0.004, p = 0.011, and p = 0.008, respectively). In contrast, we found that peri-adolescent onset cSLE (≥10 years of age) was associated with worse disease activity (SLEDAI range 0-24) (p < 0.001), higher SLICC at diagnosis (p < 0.001), as well as a higher rate of mycophenolate mofetil and hydroxychloroquine use (p = 0.003 and p < 0.001, respectively). There were no significant differences in the prevalence of neuropsychiatric symptoms or the development of Class IV/Class V lupus nephritis between the early-onset and peri-adolescent groups.

Dataset Information

Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups.

Publications

Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups.

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